| Field | Specification |
|---|---|
| Target | |
| Alternative names | Lysosomal alpha-glucosidase;3.2.1.20;Acid maltase;Aglucosidase alfa;76 kDa lysosomal alpha-glucosidase;70 kDa lysosomal alpha-glucosidase;GAA; |
| UniProt # | |
| Host | |
| Clonality | |
| Isotype | |
| Reactivity | |
| Applications | |
| Immunogen | A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids. |
| Molecular weight | |
| Purification | |
| Reconstitution | |
| Cellular localization | |
| Concentration | |
| Form | Lyophilized |
| Storage | |
| Catalog no. (Mfr.) | |
| Main SKU |
Product Overview
This rabbit polyclonal antibody recognizes lysosomal alpha-glucosidase (gene GAA) in human samples and is validated for IHC and Western blot. The predicted molecular weight is 105.3 kDa; the supplier reports observed bands at 110 kDa, 95kDa, 76kDa, 70 kDa.
Lysosomal acid α-glucosidase (GAA, acid maltase) breaks down glycogen inside lysosomes by cleaving α-1,4 and, more slowly, α-1,6 glucose linkages. Loss of GAA activity causes Pompe disease (glycogen storage disease type II), in which glycogen builds up in muscle, so the enzyme is a focus of lysosomal and neuromuscular research.
| Target | Lysosomal alpha-glucosidase (gene GAA; UniProt P10253, human) |
|---|---|
| Host / Clonality / Isotype | Rabbit / Polyclonal / Rabbit IgG |
| Reactivity | Human |
| Form | Lyophilized |
| Formulation | Per vial: 4 mg trehalose; 0.9 mg NaCl; 0.2 mg Na2HPO4; 0.05 mg NaN3 |
| Calculated MW | 105.3 kDa |
| Observed MW | 110 kDa, 95kDa, 76kDa, 70 kDa |
| Storage | As supplied: −20 °C for up to 12 months from receipt. After reconstitution: 4 °C for up to 1 month, or aliquot and keep at −20 °C for up to 6 months. Avoid repeated freeze–thaw cycles. |
Validated Applications
| IHC (paraffin sections) | 0.5–1 µg/mL (Human) |
|---|---|
| Western blot | 0.1–0.5 µg/mL (Human) |
Samples with a confirmed band (WB): human A549 cells, human HepG2 cells, human HEK293 cells, human PC-3 cells.
Recommended loading (WB): 20–40 µg of total protein per lane.
Conditions in the example images (WB): 5–20% gradient SDS-PAGE under reducing conditions, 50 µg lysate per lane, transfer to nitrocellulose membrane, blocking in 5% non-fat milk, primary antibody at 0.5 µg/mL overnight at 4 °C, HRP-conjugated secondary antibody at 1:10000, ECL detection.
Samples with confirmed staining (IHC): human liver cancer tissue, human prostatic cancer tissue.
Recommended antigen retrieval: heat-mediated, in TE buffer (pH 9.0); citrate buffer (pH 6.0) can be used instead.
Conditions in the example images (IHC): heat-mediated antigen retrieval in citrate buffer (pH 6).
Immunogen
Synthetic peptide from the middle region of human GAA. It differs from the mouse sequence at eight residues and from the rat sequence at six residues.
Reactivity Notes
The supplier lists reactivity with human. UniProt places lysosomal alpha-glucosidase in the lysosome and lysosome membrane. The samples tested by the supplier (listed above) are a practical starting point for positive controls.
Safety
Customization & Add-ons: Can’t find the antibody you need—or require a custom format for your assay? We can help you source the best match or support custom antibody solutions for diverse research needs, including species and isotype selection, conjugations and labeling (e.g., HRP/AP, biotin, fluorophores), purification grade options (Protein A/G, affinity purified), formulation preferences (buffer selection, carrier-free, glycerol-free), custom concentrations and aliquoting, low-endotoxin options for cell-based work, and application-focused QC/validation support (project dependent). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.
Michelle Eggers et al. (2021) Muscle-directed gene therapy corrects Pompe disease and uncovers species-specific GAA immunogenicity. EMBO Molecular Medicine. 10.15252/emmm.202113968
